Ontology highlight
ABSTRACT:
SUBMITTER: Medrano AZ
PROVIDER: S-EPMC2536573 | biostudies-literature | 2008 Jul
REPOSITORIES: biostudies-literature
Medrano Andrea Z AZ Barmada Sami J SJ Biasini Emiliano E Harris David A DA
Neurobiology of disease 20080407 1
A nine-octapeptide insertional mutation in the prion protein (PrP) causes a fatal neurodegenerative disorder in both humans and transgenic mice. To determine the precise cellular localization of this mutant PrP (designated PG14), we have generated transgenic mice expressing PG14-EGFP, a fluorescent fusion protein that can be directly visualized in vivo. Tg(PG14-EGFP) mice develop an ataxic neurological illness characterized by astrogliosis, PrP aggregation, and accumulation of a partially protea ...[more]